摘要Gorham病(即大块骨溶解症)是一种少见、病因不明的疾病,以骨溶解为突出表现,并因此可产生一系列并发症及临床后果.病理改变主要包括骨溶解(或被替代)及进行性、非肿瘤性的毛细血管和(或)毛细淋巴管增殖两个方面.关于前者,大多数研究者认为系破骨细胞数目增加和(或)活性增强所致;关于后者,血小板源性生长因子受体(PDGFR)信号通路机制较令人信服.以上两方面可能存在目前尚未明了的某种内在联系机制.

abstractsGorham's disease (massive osteolysis) is a rare disease of uncertain etiology. Its predom-inant manifestation is osteolysis which can cause a diverse of complications and clinical outcomes. The patho-logical features involved in Gorham's disease include mainly two aspects which may relate with each other,re-spectively the esteolysis(or the replacement of normal bone) and the aggressively expanding but non-neoplas-tic vascular or lymphatic vessel. The activation of osteoclasts is supposed to induce the former in majority's o-pinion, while concerning the etiology of the latter, the hypothesis of platelet-derived growth factor receptor sig-nal pathway seems to be relatively credible.